Clinical and immunological aspects of hyper-IgM syndrome.
Eight patients with Hyper-IgM syndrome were subjected to clinical and immunological evaluation. There were seven males and one female. All the patients had recurrent pyogenic infections; one had lymphoid hyperplasia with centrally necrotic granulomas, and one had gingivitis with neutropenia. Isohemagglutinin titers were either high or normal in all the patients and five had group 0 blood. The percentage of IgM-bearing cells were normal in five patients. The percentage of T cells were normal in all the patients, helper T cells were decreased in two patients, and suppressor T cells were increased in four patients. These results suggest that at least in some patients, the imbalances of T cell subsets may play a role in the pathogenesis of the disease rather than it being attributed to an intrinsic B cell defect.
Ersoy
F
F
Immunology Unit, Hacettepe University Institute of Child Health, Ankara.
Sanal
O
O
Tezcan
I
I
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D006942:Hypergammaglobulinemia / Q000209:etiology* / Q000276:immunology; D007075:Immunoglobulin M / Q000276:immunology*; D007223:Infant; D007958:Leukocyte Count; D008297:Male; D013577:Syndrome; D016176:T-Lymphocyte Subsets / Q000276:immunology
D007075:Immunoglobulin M